title: Treatment of severe Henoch-Schönlein and immunoglobulin A nephritis. A single center experience.
abstract: Our aim was to report the effect of two treatment regimens in 43 cases of severe Henoch-Schönlein nephritis (HSN) and immunoglobulin A nephritis (IgAN) (24 HSN, 19 IgAN). Group A, 11 HSN and 7 IgAN, 88% with an International Study of Kidney Disease in Children (ISKDC) biopsy grade > or = III and severe clinical features, were treated with corticosteroids, cyclophosphamide (CYC-P) and angiotensin-converting enzyme inhibitor/angiotensin receptor blocker (ACEi/ARB). Group B, 12 HSN and 13 IgAN, 72% with biopsy findings as above and 52% with severe clinical features, were treated with ACEi/ARB +/- corticosteroids. The outcome classification was: (a) healthy; (b) mild proteinuria, normal glomerular filtration rate (GFR); (c) active renal disease; (d) chronic renal failure. Twenty-six patients had a good outcome (a + b). The 17 children with poor outcome (c + d) had lower GFR at onset and at follow-up, higher albumin excretion at follow-up, and higher percentage of segmental glomerulosclerosis in the renal biopsy, than those with good outcome. Treatment with corticosteroids, CYC-P and ACEi/ARB was effective in increasing GFR, reducing proteinuria and decreasing the disease activity index. The proteinuria had decreased at follow-up in both groups. In group A, GFR increased and histopathological activity index declined after treatment. The outcome did not differ between groups A and B. The effects of treatment did not differ between HSN and IgAN.
authors.affiliation: Department of Clinical Science, Intervention and Technology, Division of Pediatrics, Karolinska Institutet, Karolinska University Hospital, Huddinge, 14186, Stockholm, Sweden. stella.edstrom.halling@ki.se
authors.forename: Stella
authors.initials: S
authors.lastname: Edström Halling
authors.forename: Magnus P
authors.initials: MP
authors.lastname: Söderberg
authors.forename: Ulla B
authors.initials: UB
authors.lastname: Berg
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D047228:Angiotensin II Type 1 Receptor Blockers / Q000627:therapeutic use*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D005919:Glomerular Filtration Rate; D005922:Glomerulonephritis, IGA / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D011695:IgA Vasculitis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D008137:Longitudinal Studies; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D011507:Proteinuria / Q000188:drug therapy; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D047228:Angiotensin II Type 1 Receptor Blockers; D000806:Angiotensin-Converting Enzyme Inhibitors; D003520:Cyclophosphamide; D008775:Methylprednisolone